Objectives are to understand canine globoid leukodystrophy (GLD) in relation to human GLD. In particular, we hope to explain GLD pathogenesis in morphologic terms and the nature and consequences of the enzyme defect responsible for the disease. Our current research goals are: 1) to continue maintenance of a colony of dogs for the production of GLD offspring; 2) to describe morphologic events in the pathogenesis of GLD neuropathy at the ultrastructural level; 3) to evaluate neurilemmal cell viability by cell counts in nerves with chronic Wallerian degeneration; 4) to quantify the distribution of spinal lesions in canine GLD; 5) to collaborate with Dr. Kunihiko Suzuki in evaluating enzyme activity in GLD and normal control dogs.